Abstract: PO2221
Belatacept-Induced Post-Transplant Lymphoproliferative Disorder of the Spleen That Spontaneously Resolved on Withdrawal of the Agent
Session Information
- Onco-Nephrology - 2
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1500 Onco-Nephrology
Authors
- Pennekamp, Alexander, The Christ Hospital, Cincinnati, Ohio, United States
- Pembaur, Karl Berthold, The Kidney and Hypertension Center, Cincinnati, Ohio, United States
Introduction
PTLD (Post-Transplant Lymphoproliferative Disorder) is a dreaded and serious complication of allograft transplant. Compared to CNIs, allograft rejection prophylaxis with Belatacept is less nephrotoxic, but carries a higher risk of PTLD. B-cell proliferation in PTLD is linked to EBV, and while use of Belatacept is limited to those that are EBV+ pre-transplant, prior EBV infection does not completely ensure that a patient will not develop PTLD. Patients with active PTLD characteristically show B symptoms and an increase in EBV RNA due to active viral replication. Prompt PET, BMB and blood testing must be pursued to determine a potential site of proliferation.
Case Description
64 year AAM 2 years status-post deceased-donor transplant was admitted for altered mental status, nausea, vomiting, fever of unknown origin, and weight loss. He was found to have splenomegaly on CT without other evidence of adenopathy. LDH was elevated. Patient underwent brain MRI, PET scanning, BMB and LP, all of which were normal. A full ID work-up including CMV, EBV RNA, BK were all negative. Core biopsy of the spleen demonstrated CD20/CD45+ cells with tissue effacement, suggesting a diffuse large B-cell lymphocytic infiltration/monomorphic PTLD. The sample was EBV-negative by IHC. Belatacept was withdrawn. Subsequent splenectomy was required, which showed histopathologic evidence of infarction, but complete resolution of lymphoproliferative infiltration.
Discussion
Even in EBV recipient-positive patients, there is still a risk to develop PTLD. In our case, the patient demonstrated monomorphic PTLD which resolved spontaneously when withdrawing Belatacept. This suggests that overly aggressive immune suppression, rather than induction of dysregulated proliferation, was the culprit. The fact that the patient did not show an acute increase in EBV RNA, and EBV by ISH on core biopsy of the spleen was negative, suggests that pre-transplant serosorting based on EBV seropositivity may not be sufficient to predict risk for developing PTLD. The patient had complete resolution of AMS and B symptoms with discontinuation of Belatacept, with no subsequent recurrence of PTLD after switching to sirolimus/mycophenolate.